Document Detail

Cardiac involvement in secondary haemochromatosis: a catheter biopsy study and analysis of myocardium.
MedLine Citation:
PMID:  7260965     Owner:  NLM     Status:  MEDLINE    
Although high blood transfusion regimens have improved the life expectancy of the patient with Thalassemia Major, cardiac failure and arrhythmias remain a cause of early death. It is not certain whether the massive myocardial iron deposition found in such patients is preventable by intensive chelation therapy. This study evaluates endomyocardial biopsy as a method of assessing myocardial iron deposition. Of four patients with clinical and biochemical evidence of severe haemochromatosis, only one had a myocardial iron content comparable to that found in severe haemochromatotic myocardium. The one patient with cardiac failure had an endomyocardial iron content within the normal range. Studies of the iron distribution in haemochromatotic myocardium demonstrate that the subendocardial myocardium contains only half the iron content of the subepicardial layer, and there is a large sampling variation. It is concluded that catheter endomyocardial biopsy is an insensitive method of determining early myocardial deposition because of the location of iron and the variability of the sampling. Studies of the nature of the myocardial iron protein with CM32 cation exchange resin chromatography show that there is a large increase in the haemosiderin: ferritin ratio (5:1) in iron overload myocardium as compared with the normal heart (2:1). Similar results have been observed in the liver with iron overload, where the increase in hepatic haemosiderin was associated with greater lysosomal fragility. It is possible that myocardial cell damage may also occur by the rupture of iron engorged lysosomes.
D H Fitchett; D J Coltart; W A Littler; M J Leyland; T Trueman; D I Gozzard; T J Peters
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Cardiovascular research     Volume:  14     ISSN:  0008-6363     ISO Abbreviation:  Cardiovasc. Res.     Publication Date:  1980 Dec 
Date Detail:
Created Date:  1981-10-25     Completed Date:  1981-10-25     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0077427     Medline TA:  Cardiovasc Res     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  719-24     Citation Subset:  IM    
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MeSH Terms
Cardiomyopathies / diagnosis*,  metabolism,  physiopathology
Endocardium / analysis
Enzymes / analysis
Heart Catheterization
Heart Ventricles / analysis
Hemochromatosis / diagnosis*,  metabolism,  physiopathology
Iron / analysis*
Metalloproteins / analysis
Myocardium / analysis*
Reg. No./Substance:
0/Enzymes; 0/Metalloproteins; 7439-89-6/Iron

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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